Abstract

variation of autoimmune polyendocrinopathy–candidiaxilla and pubis and is likely to be related to an asis–ectodermal dystrophy (APECED) in a series of 68 autoimmune pathogenesis (4, 6). Recently, a new patients. N Engl J Med 1990; 322: 1829–1836. autoantigen related to APS I, tyrosine hydroxylase, has 3. Wang CY, Davoodi-Semiromi A, Huang W, Connor E, been identiŽ ed in patients aVected by alopecia areata (9). Shi JD, She JX. Characterization of mutations in patients with autoimmune polyglandular syndrome type I. Hum Vitiligo, observed in 8–13% of APS I patients, appears Genet 1998; 103: 681–685. in childhood and is typically associated with the presence 4. Betterle C, Greggio NA, Volpato M. Autoimmune polyof autoantibodies directed against the SOX9 and SOX10 glandular syndrome type I. J Clin Endocrinol Metab 1997; autoantigens (1, 10). 83: 1049–1054. The high incidence of chronic mucocutaneous candidi5. Kirkpatrick CH. Chronic mucocutaneous candidiasis. J Am Acad Dermatol 1994; 31(3 Pt 2): S14–17. asis in APS type I, as Ž rst presenting feature of the 6. Delambre C, Teillac D, Brauner R, De Prost Y. syndrome, and its absence in APS types II and III Polyglandular autoimmune disease and chronic mucocutunderline the relevance of chronic mucocutaneous cananeous candidiasis. Ann Dermatol Venereol 1989; 116: didiasis as early diagnostic criteria for APS type I. In 117–121. light of this view, and related to the subsequent onset 7. De Padova-Elder SM, Ditre CM, Kantor GR, Koblenzer of the diVerent signs that complete the syndrome, PJ. Candidiasis endocrinopathy syndrome. Treatment with itraconazole. Arch Dermatol 1994; 130: 19–22. patients aVected by chronic mucocutaneous candidiasis 8. Rybojad M, Abimelec PH, Feuilhade M, Morel P, Bourrat in early childhood should have a long-term monitoring E. Familial candidiasis endocrinopathy syndrome: treatdermatological and endocrinological follow-up in order ment with  uconazole in 3 cases. Ann Dermatol Venereol to detect whether, other than the immunological defects 1999; 126: 54–56. commonly associated with this infection, an early diag9. Hedstrand H, Ekwall O, Haawik J, Landgren E, Betterle C, Perheentupa J, et al. IdentiŽ cation of tyrosine hydroxynosis of APS type I can be suspected and a correct lase as an autoantigen in autoimmune poliendocrine sindtherapy can be established (5, 8). rome type I. Biochem Biophys Res Commun 2000; 267: 456–61. 10. Hedstrand H, Ekwall O, Olsson MJ, Landgren E, Kemp REFERENCES EH, Weetman AP, et al. The transcription factors SOX9 and SOX10 are vitiligo autoantigens in autoimmune poly1. Trence DL, Morley JE, Handwerger BS. Polyglandular endocrine syndrome type I. J Biol Chem 2001; 276: autoimmune syndromes. Am J Med 1984; 77: 107–116. 2. Ahonen P, Myllarniemi S, Sipila I, Perheentupa J. Clinical 35390–5.

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